Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

T cell large granular lymphocyte leukemia (T-LGL) is characterised by semiautonomous proliferation of monoclonal cytotoxic T lymphocytes, which can result in neutropenia, splenomegaly, and is associated with various autoimmune disorders, particularly rheumatoid arthritis. The coexistence of T-LGL leukemia with B cell abnormalities has previously been identified in case reports. However, no systematic analysis to determine the frequency of this co-association has been reported. Analysis of 63 T-LGL patients revealed a frequent association with humoral immune system abnormalities. We identified coexisting B cell dyscrasias in 17 T-LGL patients (27% of total), of whom 12 had monoclonal gammopathy of unknown significance (MGUS) (19%), and 5 had chronic lymphocytic leukemia (CLL) (8%). The presence of both MGUS and CLL was found in 2 patients (3%) and follicular lymphoma was identified with MGUS in another T-LGL patient (2%). Additionally, polyclonal hypergammaglobulinemia or hypogammaglobulinemia was found in 10 additional LGL leukemia patients bringing the total frequency of B cell abnormalities in T-LGL leukemia to 43% in our cohort. The co-association of B cell pathology with T-LGL suggests that either a common antigen drives clonal B and T cells, or that humoral malignancy could serve as the stimulus for lymphocyte expansion representing an overactive anti-tumour surveillance.
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PMID:Chronic B-cell dyscrasias are an important clinical feature of T-LGL leukemia. 1846 4

A rare case of lethal idiopathic plasmacytic lymphadenopathy (IPL) with polyclonal hyperimmunoglobulinemia with chronic renal failure is described. A 40-year-old woman who had suffered from upper airways disease was admitted to the Emergency Room with acute renal failure and hypergammaglobulinemia. She developed pericardial effusion, a pruritic rash, splenomegaly and fell into a coma after 6 days. Multiple myeloma, infection, collagenopathy, and coagulopathy were ruled out. Finally, a form of malignant hypergammapathy was suspected. At autopsy, lymph nodes were infiltrated by polyclonal plasma cells and lymphocytes, with erythrophagocytosis features; immunohistochemistry confirmed the plasma cells (CD138+), with a prevalence of kappa-positive cells, B (CD20+) and T (CD3+) cells. Kidneys showed advanced glomerulosclerosis containing similar infiltrates. The cause of death was IPL with polyclonal hypergammaglobulinemia and advanced renal failure. Similar cases are unusual, and possible medical liability associated with failure to diagnose and treat idiopathic plasmacytic lymphadenopathy deserves discussion and further studies.
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PMID:A Case of Lethal Idiopathic Plasmacytic Lymphadenopathy with Polyclonal Hypergammaglobulinemia: A Medical Challenge for the Forensic Pathologist. 2736


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